Low-Flow Vascular Malformation Pitfalls: From Clinical Examination to Practical Imaging Evaluation—Part 2, Venous Malformation Mimickers

Abstract

Venous malformations are congenital endothelial malformations secondary to errors in vascular morphogenesis and are usually diagnosed in the first 2 decades of life. The clinical and imaging features of venous malformations often overlap those of other pathologic entities, creating diagnostic confusion. Furthermore, the clinical presentation and imaging appearance of venous malformations can vary, making the diagnosis challenging. Thorough knowledge of the various clinical and imaging features not only of venous malformations but also of the major potential mimic lesions is crucial for clinicians caring for patients with these lesions.

Masses of developmental and genetic origin affecting the paediatric craniofacial skeleton

“…The slow venous flow predisposes to repeated episodes of intralesional thrombosis, and sudden enlargement may occur [ 60 ]. Increased D-dimers have been reported in 42% of patients with VVMs and are highly correlated with pain caused by thrombosis [ 60 ]. Depending on patient symptoms, treatment includes multiple procedures (surgery, laser therapy and sclerotherapy).…”

“…On US, VVMs are often compressible and hypoechoic, and they present a heterogeneous sponge-like echotexture. Calcified phleboliths, although pathognomonic, are present only in 16% of cases [ 60 ]. Monophasic flow on colour Doppler US is seen in 78% of the cases, whereas absent flow due to thrombosis or sluggish flow below the limits of detection can be seen in 16% of lesions and represents a source of diagnostic confusion [ 60 ].…”

“…Calcified phleboliths, although pathognomonic, are present only in 16% of cases [ 60 ]. Monophasic flow on colour Doppler US is seen in 78% of the cases, whereas absent flow due to thrombosis or sluggish flow below the limits of detection can be seen in 16% of lesions and represents a source of diagnostic confusion [ 60 ]. Arterial flow within the VVM is identified in 6% and is either caused by facial arteries traversing the VVM or by Masson tumour [ 60 ].…”

Female Pelvic Vascular Malformations

“…On color Doppler imaging, the majority of cases have a monophasic venous waveform; however, it is common to not detect a waveform because of the sluggish flow. 25 MRI is useful to define the extent of the lesion, which may infiltrate through underlying tissue planes and muscles for a much larger area. VMs are usually isointense or hypointense on T1-weighted imaging with focal areas of decreased signal from phleboliths.…”

Reply - Management of vascular anomalies: Review of institutional management algorithm

“…This is because many other diseases may be mistaken for vascular anomalies, including plexiform neurofibromas, teratomas and soft-tissue neoplasms. [ 2 3 ] Furthermore, there is a lot of ambiguity in classification and nomenclature of these lesions. Thus, it is important that all the specialities dealing with these patients follow the recent classification system of the International Society for the Study of Vascular Anomalies 2014.…”